作者: Osama Y. Al-Dirbashi , Mohamed S. Rashed , Herman J. Ten Brink , Cornelis Jakobs , Najlaa Filimban
DOI: 10.1016/J.JCHROMB.2005.12.010
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摘要: Abstract Succinylacetone (SA) is a specific marker for the inherited metabolic disease, hepatorenal tyrosinemia. We developed stable-isotope dilution liquid chromatography tandem mass spectrometry determination of SA in dried blood spots (DBS) and urine using 13C4-SA as internal standard. was extracted, converted to butyl ester derivatized with dansylhydrazine (Dns-H). Calibration curves DBS calibrators were linear up 100 30 μM, respectively. At signal-to-noise ratio 3, limits detection 0.2 0.005 μM, Total run time 5 min. Intra- inter-assay precision expressed coefficient variation better than 9.1% more 96% recovery. The method applied retrospectively prospectively diagnosis tyrosinemia follow-up patients under treatment.